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1、硬脑膜动静脉瘘(DAVF) 发生于硬脑膜及其附属结构如静脉窦、大脑镰、小脑幕上的异常动静脉分流 约占颅内动静脉畸形的10%-15% 可见于任何年龄,成人多见第1页/共55页硬脑膜动静脉瘘(DAVF) 硬脑膜窦畸形伴动静脉瘘 新生儿或婴儿,常为巨大囊袋或硬膜湖,与其它窦或大脑静脉以缓流交通,多累及上矢状窦,常伴栓塞、闭锁或一侧颈内静脉球发育低下 婴儿型DAVF 高流速,高流量,多灶性,表现为大的窦及多发的局部动静脉瘘和大的供血血管,常继发引起皮层软膜分流,直窦常缺如;静脉出口闭塞可引起颅压增高,脑室积水 成人型DAVF第2页/共55页婴儿型DAVF 多支供血动脉 静脉窦瘤样扩张 梗塞性脑积水 直
2、窦缺如 骨皮质改变第3页/共55页女,女,1010岁岁 进行性脑神经缺失(婴儿型进行性脑神经缺失(婴儿型DAVFDAVF)CT强化: 上矢状窦扩张,脑皮质钙化,白质变薄MR T1WI:上矢状窦及窦汇巨大流空影,小脑扁桃体下移第4页/共55页成人型DAVF第5页/共55页硬脑膜动脉 前颅窝 脑膜中动脉前支 筛前、后动脉 脑膜返动脉 蝶腭动脉 中颅窝 脑膜中/副动脉 颈内动脉下外侧干 咽升动脉脑膜支 后颅窝 椎动脉脑膜支 脑膜垂体干 枕动脉脑膜支 脑膜中动脉后支 咽升动脉脑膜支 大脑后动脉分支 小脑上动脉分支 小脑下后动脉分支第6页/共55页第7页/共55页发病机制 DAVF与手术、头外伤、感染、
3、硬脑膜窦血栓形成、雌激素等因素有关,但确切发病机制不明 两种假说 “生理性动静脉交通”开放:硬脑膜动静脉之间存“生理性动静脉交通”(dormant channels)或“裂隙样血管”(crack-like vessels),某些病理状态使其开放,形成DAVF 新生血管:某些血管生长因子异常释放促使硬脑膜新生血管形成,致使DAVF形成第8页/共55页分型 按静脉引流方向分型:与临床表现及预后密切相关 按DAVF部位分型:与血供来源及治疗途径密切相关 静脉引流方向与病变部位相结合分型第9页/共55页按静脉引流方向分型Borden classificationBorden classificatio
4、n1 Venous drainage directly into dural venous sinus or meningeal vein2 Venous drainage into dural venous sinus with CVR3 Venous drainage directly into subarachnoid veins(CVR only)Cognard classificationCognard classificationI Venous drainage into dural venous sinus with antegrade flowIIa Venous drain
5、age into dural venous sinus with retrograde flowIIb Venous drainage into dural venous sinus with antegrade flow and CVRIIa+b Venous drainage into dural venous sinus with retrograde flow and CVRIII Venous drainage directly into subarachnoid veins (CVR only)IV Type III with venous ectasias of the drai
6、ning subarachnoid veinsV Venous drainage into the perimedullary plexusCVR=cortical venous reflux(可能与静脉窦闭塞有关)第10页/共55页第11页/共55页第12页/共55页按DAVF部位分型 海绵窦DAVF 横窦乙状窦DAVF 小脑幕DAVF 上矢状窦DAVF 前颅窝DAVF 边缘窦DAVF 岩上/下窦DAVF 舌下神经管DAVF第13页/共55页临床表现良性DAVF搏动性杂音眼眶充血颅神经麻痹慢性头痛无症状侵袭性DAVF颅内出血颅内高压非出血局部神经缺失血管性痴呆死亡Borden type 1C
7、ognard typeI/aBorden type 2/3Cognard type IIb-皮层静脉返流(CVR)或深静脉引流是预后不良的重要因素第14页/共55页v搏动性突眼v球结膜水肿和充血v眶周杂音v进行性视力下降v颅神经麻痹v杂音,耳鸣,头痛v眼部症状v颅内出血(少见)v杂音,耳鸣v颅内出血v中枢神经缺失v头痛v颅内出血v中枢神经缺失,痴呆v颅内出血v头痛第15页/共55页诊断 经颅多普勒:可探测血流动力学改变,特异性较低 CT与MRI:对良性DAVF敏感性较低;对侵袭性DAVF,可显示异常血管,颅内出血,局部占位效应,脑水肿,脑积水,静脉窦血栓形成及颅骨骨质异常等征象 CTA与MRA
8、:可清楚显示异常增粗的供血动脉和扩张的引流静脉及静脉窦,对瘘口位置及“危险吻合”显示欠佳第16页/共55页诊断 DSA 供血动脉 瘘口位置 引流静脉 静脉窦扩张与闭塞 脑循环异常第17页/共55页 Male,62 tentorial Male,62 tentorial DAVFDAVF(Cognard Cognard )The left lateral ICA angiogram shows a tentorial DAVF fed by an inferior marginal tentorial artery draining into a cortical veinL-ICA第18页/共
9、55页 Male,49 DAVF of anterior cranial fossa Male,49 DAVF of anterior cranial fossa (Cognard Cognard )The left lateral internal carotid arteriogram demonstrates a DAVF supplied by the anterior ethmoidal branches of the ophthalmic artery and the draining intracranial vein with a focal aneurysmal dilata
10、tion at the site of parenchymal hemorrhageL-ICA第19页/共55页tentorialtentorial DAVFDAVF(Cognard Cognard )R-ICA第20页/共55页术后1年MR示上矢状窦血栓形成,3年后自感颅内杂音,MR示脑表多发迂曲血管流空影;左侧颈外动脉造影侧位,左侧横窦DAVF伴CVR,同侧乙状窦闭塞 女,女,3737肾移植术后,左横窦肾移植术后,左横窦DAVFDAVF(Cognard a+bCognard a+b)第21页/共55页岩上窦岩上窦DAVFDAVF(Cognard )Cognard )向脊髓静脉引流向脊髓静脉
11、引流右脑膜中动脉后支,右枕动脉脑膜支及右侧脑膜垂体干供血第22页/共55页RECA造影:右侧海绵窦DAVF,引流至眼上静脉及皮层静脉男,男,5858右眼球结膜充血水肿右眼球结膜充血水肿第23页/共55页治疗 保守治疗 立体定向放射治疗 血管内介入治疗 外科手术第24页/共55页介入治疗策略 经动脉微粒栓塞(TAE-微粒):难以达到完全栓塞,通常用于缓解症状或辅助治疗 经静脉弹簧圈栓塞(TVE):治愈性手段,必须致密栓塞,否则可使症状恶化;可并发静脉壁损伤,颅内出血 经动脉NBCA/Onyx栓塞(TAE):用于复杂DAVF不能通过静脉途径栓塞时,完全栓塞率较高;可造成异位栓塞,对操作技术要求高
12、支架植入:其支撑力可恢复静脉窦正常引流并可封闭位于静脉窦壁上的瘘口;远期效果待进一步观察第25页/共55页海绵窦DAVF 保守 放疗 TAE微粒 TVE TAENBCA第26页/共55页海绵窦DAVF 经静脉途径是首选的治愈性的方法 经岩下窦入路(闭塞时亦可通过) 经眼上静脉入路 其它入路:岩上窦、对侧海绵窦、基底静脉丛第27页/共55页 Spontaneous regression of a cavernous sinus Spontaneous regression of a cavernous sinus DAVFDAVFT2WI image shows multiple flow vo
13、ids in the posterior cavernous sinus Left ECA angiogram shows a cavernous sinus dural AVF with posterior drainage into the inferior and superior petrosal sinuses Follow-up MR image shows resolution of the flow voidsL-ECAL-ECA第28页/共55页Left ECA angiogram shows a cavernous sinus DAVF draining mainly in
14、to the inferiorpetrosal sinus and pterygopharyngeal plexus Follow-up angiogram obtained 3 monthslater shows that the inferior petrosal sinus is occluded, and the dural AVFnow drains into the superior ophthalmic vein and the superficial middle cerebral vein .Althoughthe patients symptoms were unchang
15、ed, occlusion ofthe DAVF was indicatedTVE of DAVF via an occluded inferior petrosal sinusTVE of DAVF via an occluded inferior petrosal sinusL第29页/共55页Superselective venogram shows that the tip of the microcatheter has been introduced into the outlets to the superior ophthalmic vein Left CCA angiogra
16、m obtained after TVE shows complete occlusion of the DAVFTVE of DAVF via an occluded inferior petrosal sinusTVE of DAVF via an occluded inferior petrosal sinus第30页/共55页横窦乙状窦DAVF 放疗+TAE-微粒第31页/共55页横窦乙状窦DAVF TVE(可先栓塞供血动脉) 放疗+TAE-微粒 支架植入+TAE-微粒+放疗TVE避免栓塞正常皮层静脉引流系统第32页/共55页横窦乙状窦DAVF TVE(可先栓塞供血动脉) 支架植入受累
17、静脉窦及返流皮层静脉近端必须致密栓塞,以防再通致脑出血第33页/共55页横窦乙状窦DAVF TVE(手术入路、经闭塞静脉窦入路、经皮层静脉入路) TAE-NBCA 手术切除(可先栓塞供血动脉)操作难度大,要求技术高第34页/共55页The lateral left ECA angiogram shows a DAVF of the transverse sinus with CVR and occlusion of the ipsilateral sigmoid sinus. A transvenous approach via the contralateral transverse sin
18、us allowed selective catheterization of a parallel channel. Venography in this parallel channel shows the veins that were draining the fistulaConversion of an aggressive DAVF to a benign(G3) Conversion of an aggressive DAVF to a benign(G3) 第35页/共55页This parallel channel was embolized with a combinat
19、ion of platinum coils and Hydrocoil A control left ECA arteriogram shows that the CVR was eliminated, although the fistula persistsConversion of an aggressive DAVF to a benign(G3) Conversion of an aggressive DAVF to a benign(G3) 第36页/共55页The venous phase of the lateral CCA angiograms before and afte
20、r treatment, we see that these cortical veins can participate in the venous drainage of the brain after disconnection难以完全治愈时,可将侵袭性DAVF转化为良性DAVFConversion of an aggressive DAVF to a benign(G3) Conversion of an aggressive DAVF to a benign(G3) 第37页/共55页 Early arterial phase left CCA angiogram shows a t
21、ransverse-sigmoid sinus DAVF. Late arterial phase left CCA angiogram shows that the left sigmoid sinus is occluded and the dural AVF drains mainly into cortical veins and the posterior condylar vein. Superselective venogram shows a microcatheter that has been advanced via the posterior condylar vein
22、 into the affected sinusRecanalization of a transverse-sigmoid sinus DAVF after TVERecanalization of a transverse-sigmoid sinus DAVF after TVE第38页/共55页Left CCA angiogram obtained after TVE shows disappearance of the AVF. CT scan obtained 2 months after TVE shows a massive hemorrhage in the left temp
23、oral lobe. Left common carotid angiogram shows recanalization of the dural AVF at the retrograde cortical drainage outlet Recanalization of a transverse-sigmoid sinus DAVF after TVERecanalization of a transverse-sigmoid sinus DAVF after TVE可能与栓塞不致密有关第39页/共55页小脑幕DAVF 只经软脑膜静脉引流 Cognard III /IV,; Borde
24、n 3 侵袭性DAVF,颅内出血风险大 治疗难度大 老年及一般状况差的患者可考虑放射治疗第40页/共55页Treatment Options for Tentorial Dural AVFsTreatment Options for Tentorial Dural AVFsTreatment Option*Results Radiation therapy Complete occlusion (50%60%) Intervention TAE with n-butyl-2-cyanoacrylate Complete occlusion (50%100%)TVE Complete occlu
25、sion (90%100% in few case reports) Surgery (disconnection of Complete occlusion (100%)leptomeningeal venous drainage) *Surgery and TAE with n-butyl-2-cyanoacrylate are equal in terms of potential risk and technical difficulty; they are more potentially risky and technically difficult than radiation
26、therapy and less so than TVE.第41页/共55页tentorial dural AVFtentorial dural AVF (Cognard IVCognard IV)Left ECA angiogram shows a tentorial dural AVFwith leptomeningeal-cortical venous drainage and venous ectasia Lateral radiograph shows the plannedradiation field Left CCA angiogram obtained 8 months af
27、ter radiation therapyshows complete obliteration of the tentorial dural AVF第42页/共55页 Male,62, presented with a brain stem hemorrhage Male,62, presented with a brain stem hemorrhageThe left ICA angiogram shows a DAVF fed by an inferior marginal tentorial artery draining into a cortical vein. Using a
28、transvenous approach catheterization of the venous pouch was feasible. Coils were deposited within the cortical vein and the venous pouch 第43页/共55页上矢状窦DAVF 发生与上矢状窦血栓形成密切相关 经静脉途径栓塞困难,常需经手术入路静脉窦栓塞或手术治疗 部分病例(瘘口较大)可经动脉行静脉窦栓塞(静脉窦无正常静脉引流)第44页/共55页Treatment Options for Superior Sagittal Sinus Dural AVFsTre
29、atment Options for Superior Sagittal Sinus Dural AVFsTreatment Option* Results Radiation therapy Unknown Intervention TAE with particles Complete occlusion (rare) TVE Complete occlusion (90%100%) TAE with n-butyl-2-cyanoacrylate Complete occlusion (90%100%) Transarterial sinus catheterization Comple
30、te occlusion (100% in case and coil embolizatio reports) Surgery (sinus isolation or resection) Complete occlusion (90%100%) combined with intervention *Treatment options in decreasing order of potential risk and technical difficulty are TAE with n-butyl-2-cyanoacrylate,surgery, TVE, and radiation t
31、herapy.第45页/共55页Superior sagittal sinus dural AVF Superior sagittal sinus dural AVF Right ECA angiogram shows a dural AVF with cortical reflux and occlusion of the superior sagittal sinus Right ECA angiogram obtained during transarterial sinus embolization shows a microcatheter that has been advance
32、d into the superior sagittal sinus via the right middle meningeal artery Right ECA angiogram obtained after embolization shows obliteration of the AVF第46页/共55页前颅窝DAVF 多由双侧眼动脉的筛动脉供血 经软脑膜静脉引流 Cognard III /IV; Borden 3 侵袭性DAVF,颅内出血风险大 外科手术相对安全,疗效好第47页/共55页Treatment Options for Anterior Fossa Dural AVFs
33、Treatment Options for Anterior Fossa Dural AVFsTreatment Option* ResultsRadiation therapy UnknownIntervention TAE with n-butyl-2-cyanoacrylate Complete occlusion (90%100% in a few case reports) TVE with a retrograde cortical Complete occlusion (90%100% in venous approach a few case reports)Surgery (disconnection of Complete occlusion (100%)leptomeningeal venous drainage) *TVE and TAE with n-butyl-2-cyanoacr
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